Pain, Fever, and ACS in Sickle Cell Disease Sirish Palle, M.D. July 26, 2011.
Post on 20-Jan-2016
214 Views
Preview:
Transcript
Pain, Fever, and ACS in Sickle Cell Disease
Sirish Palle, M.D.
July 26, 2011
Adenine to thymidine at codon 6 in beta Hb on Chromosome 11 leads to substitution of glutamic acid by valine
Identification
Alabama newborn screen tests for sickle cell disease If a screen is positive, order Hb electrophoresis
for verification
If a child has trait, counsel the family regarding risk in future children
Check Hb electrophoresis at one year old
Newborns
If diagnosed with sickle cell disease: Start prophylactic PCN VK at 125 mg PO BID
before 2 months old to decrease risk of death from pneumococcal infection
Refer to Hematology, refer family to Genetics for counseling
Give Prevnar (pneumococcal conjugate vaccine) at 2, 4 and 6 mos old
Give influenza vaccine at starting at 6 mos
Infants and Toddlers
Start Folic Acid 1 mg PO daily by one year old Give Prevnar (conjugate) at 15 mos Give Pneumovax (polysaccharide) at 2 yo & 5 yo Those that have already received their 4 doses
of Prevnar 7, get additional dose of Prevnar 13 Continue to vaccinate yearly against influenza At 3 yo, change to PCN VK 250 mg PO BID May stop PCN VK at 5 yo ≤16yo need TCD to assess for risk of CVA and
need for chronic transfusion to prevent
Outcomes
Severity is generally based on Hb variant: SS > S-betao thalassemia > S-beta+ thalassemia > SC
Median life expectancy for HbSS is 45 years, for HbSC is 65 years
High WBC, low Hb (<7), and VOC crisis in the first year of life are the known risk factors for severe morbidity
Vaso-Occlusive Crises
AKA Pain Crisis
VOC (Vaso-Occlusive Crisis)
Triggers: Infection Temperature extremes Dehydration Stress Idiopathic
History to obtain
Location of pain, severity on 0/10 scaleIs this their typical pain or is it different?Do they have significant headache (worry
about stroke)?Do they have SOB, chest pain, cough
(worry about ACS)?If they are male, do they have priapism?Do they have a fever (worry about ACS,
sepsis, osteomyelitis)?
Labs to order
CBC with differential Don’t be surprised if the WBC count is elevated
due to stress response
Reticulocyte count Expect it to be elevated
Chem 14
Therapies
Hydration: D5 ½ NS with 20 meq KCl/L to run at 150% maintenance (unless concerns for ACS or for long periods of time)
Heating packsPT consult for ROM, TENS unit, whirlpoolEncourage OOB, incentive spirometry
Oral Pain Medications
Acetaminophen with Codeine: 120 mg/12 mg/5 mL; 0.5 mg/kg/dose of codeine PO Q 4-6 hours; max dose 60 mg codeine per dose; watch acetaminophen component
Acetaminophen/Hydrocodone (Lortab): 500 mg/7.5 mg/15 mL; dose depends on weight, from 3.75 mL to 15 mL PO Q 4-6 hours; watch acetaminophen component
Parenteral Pain Medications
Ketorolac (Toradol): NSAID 0.5mg/kg IV/IM Q6 hours, max dose 30 mg IM,
15 mg IV for pediatrics Don’t use if concerns for bleeding Don’t use with other NSAIDS Caution if renal impairment Watch for gastric irritation Use for 5 days only
Parenteral Pain Medications
Nalbuphine (Nubain): opioid agonist/antagonist 0.1-0.3 mg/kg/dose IV Q 2-4 hours Max dose 20 mg Monitor for respiratory depression Good for patients who have pruritis with
morphine
Morphine
Parenteral 0.1-0.2 mg/kg/dose SC/IM/IV Q2-4 hours Max dose 15 mg Can give via PCA if 5 years or older, depends
on child’s maturity Counsel family that they cannot push the button! This
counteracts the built in safety of a PCA.
Morphine
Oral MS Contin (extended release)
0.3-0.6 mg/kg PO Q 12 hours Don’t chew Comes as 15,30,60 mg
MSIR (immediate release) 0.2-0.5 mg/kg PO Q 4-6 hours PRN severe pain Comes as 15 mg, 30 mg pills, or as 10 mg/5mL, 20
mg/10 mL solution
Transition from IV to PO Morphine
Oral dose is 3x the IV dose If a patient is comfortable on Morphine 3 mg IV
Q 3 hours, total daily dose is 24 mg/day Oral dose = 3 x 24 = 72 mg/day Can give MS Contin 30 mg PO Q12 hours Can give MSIR for breakthrough pain; 3-5 mg
PO Q 4-6 hours PRN severe pain
Other Medications
If patient is on hydroxyurea at home, continue it at the same dose
Folate 1 mg PO daily
Consider transfusion in the case of severe anemia; typically not done in VOC unless acute chest, risk of stroke, acute multi-organ failure, heart failure, reticulocytopenia (eg ParvoB19), hepatic/splenic sequestration or preoperative
Fever
Fever Functional asplenia from sickling in the
microcirculation in 90% of patients by age 6 years unless chronic transfusions which may prolong the
function of the spleen Increased risk of infection with encapsulated
organisms Haemophilus influenzae type b Streptococcus pneumoniae Salmonella typhi Neisseria meningitides Group B streptococcus Klebsiella pneumoniae
Bottom Line
Any patient with sickle cell disease and T>101 F (38.4 C) gets parenteral antibiotics, even if you have a source!
Labs that you must have
CBC with differentialReticulocyte countCRPBlood cultureChest X-ray
Labs that you might want
CP14UA, urine cultureParvovirus B19 titers if a drop in Hb and
inappropriately low reticulocyte count Look for elevated IgM, if +, needs isolation from
pregnant women
If swollen painful limb, consider osteomyelitis (MRI)
Medications
Ceftriaxone (Rocephin): 50-75 mg/kg/day IV divided Q12-24 hours 75-100 mg/kg/day IV divided Q12-24 hours if cephalosporin resistant S
pneumo suspected/prevalent in area
Vancomycin: Consider adding if clinically ill Commonly used vancomycin doses outside of the neonatal period range
from 40 to 60 mg/kg/d divided every 6 to 8 hours The exact dose will depend on the clinical presentation and may need to
be altered based on the results of therapeutic monitoring Level prior to 5th dose – trough Some evidence suggests that keeping vancomycin trough levels >10
μg/mL may help prevent the development of heteroresistance in MRSA, level closer to 15-20 in CNS, bone/joint infections and/or suspected MRSA pulmonary infections
Pediatric Infectious Disease Journal:May 2010 - Volume 29 - Issue 5 - pp 462-464
Adjusting Vancomycin Overall, nephrotoxicity in adult patients is estimated to be <5% However, there are reports of an increased risk of nephrotoxicity when
vancomycin is combined with an aminoglycoside Use with caution in renal insufficiency (BUN/Creat) & GFR
Although initially reported to be associated with ototoxicity, this association has been called into question
Many initial reports of ototoxicity occurred in patients with potential confounding factors (eg, concurrent meningitis and aminoglycoside use)
Red Man's Syndrome an association of erythema and flushing of the face, neck, and torso occurs in a significant number of patients (up to 50% in some series) and is believed to be secondary to histamine release stop/slow infusion rate and administer Benadryl
Other Medications
Hydroxyurea: Continue at the patient’s home dose Hold if leukopenia, neutropenia, or
thrombocytopenia is noted
Folate 1 mg PO daily
Acute Chest Syndrome
AKA new infiltrate on CXR and a fever
Acute Chest Syndrome
Definition is debated… New infiltrate on CXR (the best film is an old film) AND one or
more of the following: Fever (>38.5) Chest pain Tachypnea, wheezing, cough or appearance of increased work
of breathing (retractions) Hypoxemia relative to baseline measurements
Bottom Line: keep ACS forefront in your mind if your patient has any pulmonary complaints: cough, SOB, chest pain, chest wall pain, hypoxia on exam
Febrile pt with SCD need xray up to 61% not clinically suspected prior to radiograph evidence of infiltrate
Repeat CXR on kids in VOC pain crisis if resp symptoms
Treatment
Incentive spirometry with older patients (10 breaths per hour while awake) or have younger children blow bubbles
Albuterol nebulizer/MDI Q 4 hours ATC Chest PT, Mucomyst nebs Ceftriaxone IV or other 3rd generation Cephalosporin
(consider S pneumo resistance)
Azithromycin 10mg/kg PO/IV day one, then 5 mg/kg daily for days 2-5
Continue Folate and Hydroxyurea
Treatment
When should you give oxygen? When your patient has desaturations <90-92% Starting oxygen when not needed ( for
“comfort”) can decrease reticulocytosis If you have to start oxygen, get an ABG first
unless oxygen need is urgent Keep O2 Sats <95% once O2 started
Treatment
When should you give blood? Keep H/H around 10/30 Transfuse with leukocyte poor, sickle trait negative
PRBCs; consider extended antigen match in chronic transfusion patients to avoid alloimmunization
premedicate with Tylenol and Benadryl Do not transfuse to >12/36 as this can increase the risk
for stroke Consider exchange transfusion if worsening even with
H/H 10/30 Need double lumen central line; Red Cross involved
Consent for Blood
Hydration Status
Overhydration can worsen ACS If dehydrated, correct with isotonic solution D5 ½ NS with 20 meq KCl/L at 100%
maintenance for continued fluid needs Daily weights
Monitor in and outs closely Adjust fluids as needs Furosemide if fluid overload suspected
Remember…
Children with sickle cell disease and acute chest syndrome can get ill very quickly and go into respiratory failure
When called to evaluate children with sickle cell disease with shortness of breath, ALWAYS go to the bedside to assess them
Case 1
Kevin is a 13 yo AAM with Hb SS disease who is admitted with left leg pain for the last 2 days, unresponsive to Lortab 5mg at home. Pain is 7/10, nonradiating, and is similar to previous pain crises.
History
What further questions? Fever? SOB? Cough? Priapism? HA? Injury or wounds?
PE
Well developed, NAD, mild scleral icterusT=99, HR=80, RR=16, BP 110/70, O2 sats
97% RA1/6 SEMLeg without erythema or wounds, mild
TTP, CR brisk
Labs
H/H 10/30Retic count 5%CP14 with T bili mildly elevated
What medications?
He weighs 45 kg and has no allergies
What else do you want to order to treat the pt in the hospital?
Hospital course
Hospital Day # 2: You are called at 0200; his leg pain is 2/10 but he has abdominal pain. What do you want to do?
Hospital Day # 3: his pain is 2/10 and he is tolerating food well. What do you want to do?
Hospital Course
His leg and abdominal pain resolves and you discharge him home.
What advice should you give his parents? Importance of yearly influenza vaccine Importance of fever Folate 1 mg PO daily Pain management and concerns for addiction
Case 2
Alexis is a 2 yo AAF with HbSS disease who comes to your office as a new patient with T=102. Her parents report that she has had rhinorrhea for 3 days and has been pulling at her left ear. She has been eating and drinking well and has normal UOP. You examine her and diagnose left otitis media.
What do you want to do?
Admit her to the hospitalLabs?Medications? She weighs 15 kg and has
no allergies.
On call
You are paged at 0100 on hospital day #2. Alexis has vomited once and her parents say that she is not acting right. The nurse reports that she seems a little sleepy, but it is past her bedtime.
What do you do?
In her room…
You find Alexis to be sleepy but awakes easily. She is fussy when you awaken her and is somewhat consolable by her mother. T=104.3, BP is 85/50, O2 sats=100% RA, RR=30. PE: CR is 2-3 seconds, lungs are clear.
What do you do?
Follow-up
A nurse calls you with Alexis’s Vancomycin level of 14 at 2100 the next night. She is worried.
What do you do?What if the level was 22?What if the level was 4?
Discharge Advice
Yearly influenza vaccineHas she had Pneumovax (due at 2 yo)?PCN VK 125 mg PO BIDFolate 1 mg PO dailyFollow up in your office
Case 3
Tony is a 16 yo AAM with HbSS disease who comes into the ER with a cough for 24 hours and pain in his left chest.
He denies fever at home, denies SOB, denies priapism. He is on hydroxyurea at home. He has received multiple transfusions.
What next?
Physical examAre there crackles? Is he tachypneic? Is
he febrile? What is his O2 sat?
CXR: left lower lobe infiltrate
What next?
Medications? He weighs 70 kg and has no allergies
Other therapies?
top related